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Alpha-1 antitrypsin deficiency (AATD) is an inherited condition that causes AAT proteins, which protect the lungs and other organs, to get trapped in the liver. This increases risks of both lung and liver disease, and it can cause early-onset emphysema.

AATD occurs most often in white people of European descent, although it can occur in any ethnic group. Estimates of people with AATD range from about one in every 1,600 to about one in every 5,000. Because people with AATD don’t always develop serious complications, they might not even know they have it.

The first symptoms of AATD usually occur between ages 20 and 40. Symptoms can include shortness of breath, wheezing, and reduced physical endurance, as well as lung infections, vision problems, tiredness, weight loss, and a rapid heartbeat upon standing.

People with severe AATD can develop emphysema early in life (between ages 40 and 50). Emphysema causes a chronic cough, trouble breathing, and wheezing, which is why AATD is often misdiagnosed as asthma.

Other potential complications of AATD include cirrhosis and other liver diseases, often diagnosed in infancy and early childhood. Rarely, people with AATD can develop a painful skin disease called necrotizing panniculitis.

People with AATD are more susceptible to life-threatening lung diseases. Smoking and exposure to tobacco smoke are the most significant risk factors. Exposure to toxic substances, dust, and fumes are also risk factors.

People with AATD inherit the condition from their parents. The most common defective genes tied to AATD are known as S and Z, while M is considered normal. So, a person without AATD will have two M genes (MM). Generally, people with one faulty AAT gene are carriers of the disorder, while people with two faulty AAT genes have the disorder. However, even people with two faulty genes might never have complications or even know they have AATD. The table below shows how AAT genes relate to circulating AAT protein levels and associated lung disease.

AAT Gene Diagnosis Serum AAT Levels: Risk of Emphysema
μM mg/dL
MM Normal 20-48 150-350 Normal (low)
MS Carrier Unknown Unknown Low
MZ Carrier 17-33 90-120 Possibly increased
SS AAT Deficient 15-33 100-200 Unknown
SZ AAT Deficient 8-16 75-120 Likely increased
ZZ AAT Deficient 2.5-7 20-45 Definitely increased
Z-Null AAT Deficient Unknown Unknown Definitely increased
Null-Null AAT Deficient 0 0 Definitely increased

There is no cure for AATD, but the related lung diseases can be treated. Importantly, not smoking (or quitting smoking) will help delay or prevent lung diseases. A healthy diet and a suitable level of regular physical activity will help maintain lung function.

Alpha-1 augmentation therapy might be prescribed in severe cases to help slow a decline in lung function. Augmentation therapy is the infusion of an alpha-1 proteinase inhibitor (A1Pi) into the bloodstream. This can be administered safely at home with the help of a nurse. Diplomat Specialty Infusion Group offers multiple brands of A1Pi treatments—including Aralast®, Glassia®, and Zemaira®—and can provide qualified in-home nursing service.

Source: National Heart, Lung, and Blood Institute

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How We
Help

In-Home Care

We offer in-home services to make care more convenient for patients and reduce the burden on caregivers. We are passionate about adherence and want our patients to have successful treatments; in-home care reduces costs and helps patients maintain independence.

Shipping

We do everything we can to ensure the right medications arrive on time in the right place. Diplomat coordinates with a third-party carrier to facilitate delivery per patient’s or prescriber’s instructions.

Education

At Diplomat, we want our patients to have everything they need to feel supported. That’s why we provide disease–state specific education materials to keep them and their loved ones informed.

Advocacy

We understand that navigating the complex health care system can be a burden. We try to lighten it by providing advocacy services that offer personalized help when patients need it. If necessary, our patients receive prior authorization and appeals assistance. Our benefits investigation team helps them every step of the way.

Products We Support

Featured Technology

BioFeedback is a health outcomes reporting system that provides physicians who prescribe alpha-1 augmentation therapy with quarterly reports of patient treatment outcomes, including:

  • Drug dispense and medical event history
  • FEV1 (in mL)
  • FEV1/FEV1 predicted
  • FEV1/FEV6

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Return to homepage

How We
Help

In-Home Care

We offer in-home services to make care more convenient for patients and reduce the burden on caregivers. We are passionate about adherence and want our patients to have successful treatments; in-home care reduces costs and helps patients maintain independence.

Shipping

We do everything we can to ensure the right medications arrive on time in the right place. Diplomat coordinates with a third-party carrier to facilitate delivery per patient’s or prescriber’s instructions. We don’t let anything get between a patient and their treatment.

Education

At Diplomat, we want our patients to have everything they need to feel supported. That’s why we provide disease–state specific education materials to keep them and their loved ones informed.

Advocacy

We understand that navigating the complex health care system can be a burden. We try to lighten it by providing advocacy services that offer personalized help when patients need it. If necessary, our patients receive prior authorization and appeals assistance. Our benefits investigation team helps them every step of the way.

Products We Support

Take the first step in partnering with us.

Return to homepage

How We
Help

In-Home Care

We offer in-home services to make care more convenient for patients and reduce the burden on caregivers. To help patients maintain independence, we provide in-home nurses and training for those who wish to self-administer treatments outside of a clinical setting.

Shipping

We do everything we can to ensure the right medications arrive on time in the right place. Diplomat coordinates with a third-party carrier to facilitate delivery according to your instructions or those of your doctor.

Education

At Diplomat, we want our patients to have everything they need to feel supported. That’s why we provide disease–state specific education materials to keep you and your loved ones informed.

Advocacy

We understand that navigating the complex health care system can be a burden. We try to lighten it by providing advocacy services that offer personalized help when patients need it. If you need copay assistance, our funding team goes to work, connecting you with the third-party organizations that can help. We’re here for you every step of the way.

Products We Support

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